Pheochromocytoma and Paraganglioma: Risk Factors

Approved by the Cancer.Net Editorial Board, 06/2020

ON THIS PAGE: You will find out more about the factors that increase the chance of developing a pheochromocytoma or paraganglioma. Use the menu to see other pages.

A risk factor is anything that increases a person’s chance of developing a tumor. Although risk factors often influence the development of a tumor, most do not directly cause it. Some people with several risk factors never develop a tumor, while others with no known risk factors do. Knowing your risk factors and talking about them with your doctor may help you make more informed lifestyle and health care choices.

What causes pheochromocytomas and paragangliomas is unknown, and no avoidable risk factors have been found. However, the following factors may raise a person's risk for these types of tumors:

  • Inherited syndromes and gene changes. Between 25% and 35% of pheochromocytomas and paragangliomas are linked to hereditary syndromes. Changes to certain genes have also been associated with the tumors when they are not connected to known hereditary syndromes. Syndromes and gene changes that raise the risk of developing a pheochromocytoma or paraganglioma include:

    • Multiple endocrine neoplasia type 2 (MEN2). Approximately 50% of people with MEN 2 develop a pheochromocytoma.

    • Von Hippel-Lindau syndrome (VHL). Approximately 10% to 20% of people with VHL will develop a pheochromocytoma. They often occur in both adrenal glands.

    • Neurofibromatosis type 1 (NF1). Approximately 5% of people with NF1 are diagnosed with a pheochromocytoma or paraganglioma.

    • Succinate dehydrogenase (SDHx) syndromes. Germline, or inherited, mutations in the SDH family of genes have also been associated with an increased risk of pheochromocytoma and paraganglioma. The Carney-Stratakis dyad, for example, is linked with paragangliomas, gastrointestinal stromal tumorrenal cell (kidney) carcinoma, and pituitary adenoma.

    • Other mutations. There are many other, less common genetic mutations that may increase the risk of pheochromocytoma or paraganglioma.   

  • Age. A pheochromocytoma can occur at any age, but the tumor is most common in people between ages 30 and 50.

  • Gender. Men and women have an equal chance of developing a pheochromocytoma.

  • Race/ethnicity. People of all races can be diagnosed with a pheochromocytoma, but the tumor is less common in African Americans.

  • Carney triad. This rare, non-hereditary condition can cause paragangliomas, GISTs, and tumors in the cartilage of the lungs to form. It almost exclusively affects women. 

Researchers continue to look into what factors cause pheochromocytomas and paragangliomas and what people can do to lower their personal risk. Talk with your health care team if you have concerns about your personal risk of developing these types of tumors.

The next section in this guide is Symptoms and SignsIt explains what body changes or medical problems a pheochromocytoma or paraganglioma can cause. Use the menu to choose a different section to read in this guide.